Aggression and Psychiatric Comorbidity in Children With Hypothalamic Hamartomas and Their Unaffected Siblings

Weissenberger, Anne Austin; Dell, Mary Lynn; Liow, Kore; Theodore, William H.; Frattali, Carol; Hernandez, Daphne C.; Zametkin, Alan J.

doi:10.1097/00004583-200106000-00015

Cited by 120 publications

(74 citation statements)

References 32 publications

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“…95 Weissenberger et al 116 evaluated 12 children between 3 and 14 years of age, along with the parents and age-matched siblings. They noted that children with HH and gelastic seizures had a significantly higher incidence of psychiatric comorbidities, such as oppositional defiant disorder (83.3%), attention deficit-hyperactivity disorder (75%), conduct disorder (33.3%), and affective disorders (16.7%).…”

Section: Psychiatric Disordersmentioning

confidence: 99%

“…The true prevalence of epilepsy due to HH remains unknown, but is estimated to be 1-2 per 100,000. 116 In addition to gelastic seizures, a hallmark of HH-related epilepsy, the syndrome is also commonly characterized by other types of intractable seizures. Also, patients with HH often display severe cognitive impairment, pervasive developmental disorders, psychiatric disorders, and other major behavioral disturbances.…”

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confidence: 99%

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Hypothalamic hamartomas. Part 1. Clinical, neuroimaging, and neurophysiological characteristics

Mittal

Montes

et al. 2013

FOC

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Hypothalamic hamartomas are uncommon but well-recognized developmental malformations that are classically associated with gelastic seizures and other refractory seizure types. The clinical course is often progressive and, in addition to the catastrophic epileptic syndrome, patients commonly exhibit debilitating cognitive, behavioral, and psychiatric disturbances. Over the past decade, investigators have gained considerable knowledge into the pathobiological and neurophysiological properties of these rare lesions. In this review, the authors examine the causes and molecular biology of hypothalamic hamartomas as well as the principal clinical features, neuroimaging findings, and electrophysiological characteristics. The diverse surgical modalities and strategies used to manage these difficult lesions are outlined in the second article of this 2-part review.

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Section: Psychiatric Disordersmentioning

confidence: 99%

mentioning

confidence: 99%

Hypothalamic hamartomas. Part 1. Clinical, neuroimaging, and neurophysiological characteristics

Mittal

Montes

et al. 2013

FOC

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“…When epilepsy begins later, learning and behavioral problems are fewer, gelastic seizures are less prominent, and the epilepsy syndrome is usually restricted to 1 or 2 partial seizure types. 22,34,35,41 The majority of the cases of hamartoma reported were drug refractory, and the resection or disconnection allowed good results and a low complication rate, if surgery was tailored to the anatomical characteristics of the individual patient. According to the classification proposed by Delalande and Fohlen, 7 which categorized HHs into 4 types, surgical outcome is good in patients with Types I, II, and III (Type I, horizontal insertion plane, lesion may be unilateral; Type II, vertical insertion plane and intraventricular location; and Type III, combination of Types I and II), but not good (low rate of seizure freedom) in patients with socalled giant hamartomas (Type IV).…”

Section: Discussionmentioning

confidence: 99%

Deep brain stimulation for the treatment of drug-refractory epilepsy in a patient with a hypothalamic hamartoma

Marras

Rizzi²,

Villani

et al. 2011

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Hypothalamic hamartomas (HHs) are developmental malformations associated with a range of neurological problems, including intractable seizures. There is increasing evidence of the epileptogenicity of the hamartoma and of the inhomogeneous distribution of the epileptic abnormalities within the malformation. The management strategy for treatment and results differ according to the insertion plane and the extension of the malformation into the hypothalamus. Cases characterized by extensive involvement of the hypothalamus are particularly challenging. The authors describe the case of a patient with drug-resistant epilepsy and a large hypothalamic hamartoma with an extensive area of attachment. The patient underwent implantation of 2 deep brain electrodes. The intraoperative recording showed a synchronous interictal epileptic discharge in the left temporal lobe and on the left side of the lesion. The patient was treated with chronic high-frequency stimulation. No side effects due to the stimulation were reported. At 18 months' follow-up, a reduction in complex partial seizure frequency was reported, but no significant reduction in overall seizure frequency was noticed (p = 0.14, t-test). The authors report on neurophysiological studies of the relationship between HH and epilepsy, and also discuss the literature on chronic high-frequency stimulation, including its rationale and the results of chronic stimulation of various targets for the treatment of drug-resistant epilepsy due to HH.

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“…Owing to significant, but partial, efficacy 25 patients (62.5 %) were treated twice. The preoperative cognitive deficits, behavioral disturbances, and investigated relationship of seizure severity and anatomical type to cognitive abilities were characterized [71,72]. Low peripheral doses were used because of the close relationship with optic pathways and hypothalamus (median 17 Gy; range 13-26 Gy).…”

Section: Epilepsymentioning

confidence: 99%

Gamma Knife for Functional Diseases

Régis

2014

Neurotherapeutics

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Aggression and Psychiatric Comorbidity in Children With Hypothalamic Hamartomas and Their Unaffected Siblings

Cited by 120 publications

References 32 publications

Hypothalamic hamartomas. Part 1. Clinical, neuroimaging, and neurophysiological characteristics

Hypothalamic hamartomas. Part 1. Clinical, neuroimaging, and neurophysiological characteristics

Deep brain stimulation for the treatment of drug-refractory epilepsy in a patient with a hypothalamic hamartoma

Gamma Knife for Functional Diseases

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